成人重症肌无力眼肌型的临床特征

doi:10.3760/cma.j.cn115909-20201124-00453

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摘要目的：分析成人重症肌无力眼肌型（OMG）患者的临床特征。方法：回顾性系列病例研究。收集2016年6月至2019年10月暨南大学附属第一医院眼科收治的40例成人OMG患者的病例资料，并对病程、性别比例、眼部表现、复视特征、眼外肌麻痹情况和辅助检查结果等进行分析。结果：40例成人OMG患者中男女比例为1∶1。病程中位数6个月（范围：1个月～10年）。27例表现为单纯复视（68%），9例表现为单纯上睑下垂（22%），4例表现为复视合并上睑下垂（10%）。31例复视患者中，水平复视20例（64%），垂直复视11例（35%），所有复视患者均表现为双眼多条眼外肌呈不同程度麻痹。31例复视患者中主要受累眼外肌分布：外直肌16例（52%），上直肌6例（19%），内直肌4例（13%），下直肌3例（10%），上斜肌1例（3%），下斜肌1例（3%）。辅助检查显示新斯的明试验阳性32例（80%），乙酰胆碱受体抗体阳性4例（10%），合并胸腺异常5例（13%），肌电图异常2例（5%），合并甲状腺异常19例（48%）。结论：成人OMG临床表现多以水平复视为首发症状，其中外直肌受累较为常见。

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关键词 ： , 重症肌无力眼肌型, 复视, 眼肌麻痹, 新斯的明试验, 成人

Abstract： Objective: To analyze the clinical features and ophthalmoplegic characteristics of ocular myasthenia gravis (OMG) in adult patients. Methods: A retrospective case series study of the medical records of 40 adult patients diagnosed with OMG at the First Affiliated Hospital of Jinan University from June 2016 to October 2019 was performed to analyze the clinical features of OMG. The descriptive statistics included the duration of symptoms, gender distribution, ocular symptoms, diplopia features, ophthalmoplegic characteristics and the results of diagnostic tests. Results: Of the 40 adult OMG patients, 50% of the cases (20) were men. The median course of the disease among all patients was 6 months (range: 1 month to 10 years). At the first visit, 27 patients (68%) showed diplopia only, 9 patients (22%) showed ptosis only, 4 patients (10%) showed diplopia with ptosis. Among the patients with diplopia (n=31), 20 cases (64%) showed horizontal diplopia and 11 cases (35%) showed vertical diplopia. Multiple binocular extraocular muscles were affected in all diplopia patients. The most paralytic extraocular muscle was the lateral rectus (16 cases, 52%), and in descending order, the superior rectus (6 cases, 19%), medial rectus (4 cases, 13%), inferior rectus (3 cases, 10%), superior oblique (1 case, 3%), and inferior oblique (1 case, 3%). A neostigmine test was positive in 32 of 40 patients (80%), acetycholine receptor antibody assay was positive in 4 of 40 patients (10%), abnormal electromyography results were found in 2 of 40 patients (5%), thymus CT showed abnormal findings in 5 of 40 patients (13%), and 19 cases (48%) combined the thymus with thyroid disorders. Conclusions: Horizontal diplopia was the initial symptom in most adult OMG patients and the lateral rectus was the most susceptible muscle.

Key words： ocular myasthenia gravis diplopia ophthalmoplegia neostigmine test adult

收稿日期: 2020-11-24

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通讯作者: 周清（ORCID：0000-0002-3505-1989），Email：kerryzh@163.com

引用本文:

唐浚杰陈芳圆陈剑杨媛婷周清. 成人重症肌无力眼肌型的临床特征[J]. 中华眼视光学与视觉科学杂志, 2021, 23(8): 604-609. Junjie Tang, Fangyuan Chen, Jian Chen, Yuanting Yang, Qing Zhou. Analysis of the Clinical Features of Adult Ocular Myasthenia Gravis. Chinese Journal of Optometry Ophthalmology and Visual science, 2021, 23(8): 604-609. DOI: 10.3760/cma.j.cn115909-20201124-00453

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https://www.cjoovs.com/CN/10.3760/cma.j.cn115909-20201124-00453 或 https://www.cjoovs.com/CN/Y2021/V23/I8/604

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